Failure to thrive (FTT) refers to failure of expected weight gain, striking lack of well-being and inadequate physical growth in children. The causes vary with geographical and socio-economic factors. In developed countries, FTT is usually a symptom of an underlying disease, often a gastrointestinal or neurological disorder. However, in developing countries, FTT is often associated with inadequate caloric intake and malnutrition. Such children are at an increased risk of infections and infection-related mortality which may be related to altered immune responses. Rarely some Primary immunodeficiencies (PIDs) can manifest as FTT. Not much data regarding neutrophil functions in these children is available. The present study aimed to analyse the functional activity of neutrophils in children with FTT using a highly sensitive and specific flow cytometry-based assay. 25 children with FTT (up to 5 years) and 25 healthy controls were assessed for haematological parameters and neutrophil oxidative burst activity by DHR Assay using Flow cytometry. Compared to controls, the cases had significantly lower haemoglobin, hematocrit, RBC count and MCHC but a higher eosinophil count (P<0.0001). On flow cytometry, the Neutrophil Oxidative Index (NOI) was significantly reduced in cases (P<0.0001). 1 of 25 cases (4%) showed no change in neutrophil fluorescence after stimulation, suggesting the presence of CGD, which was later confirmed with molecular assay revealing a CYBB mutation. To conclude, children with FTT have a decreased Neutrophil Oxidative Burst, suggesting defective killing of pathogens by phagocytes. Also, the presence of CGD should be ruled out in such children.

Glucose 6-phosphate dehydrogenase (G6PD) activity of red blood cells (RBC) may be helpful as a prognostic factor and a probable predictive indicator of disease activity in children with acute lymphoblastic leukemia (ALL). This cross-sectional, case-control study was performed on almost 133 pediatric ALL cases from 2016 to 2020 in an oncology hospital. Patients with a history of blood transfusion within the last three months, acute hemolytic crisis, any other type of enzyme deficiency like pyruvate kinase and hexokinase, and chronic liver disease were excluded. The G6PD activity in RBC was measured using the spectrophotometric method. In addition, the G6PD activity was assessed in 133 normal individuals as a control group. According to the kit, the G6PD <1.5 IU/g of Hb level was recognized as severely deficient. The correlation of G6PD activity with disease activity and other parameters in ALL patients was determined using the Pearson correlation test. Data were measured by an independent t-test and a one-way ANOVA test. The mean G6PD activity of RBC in the control (n=133) and patient group (n=128) was 9.1±2.08 IU/g of Hb and 11.12±3.8 IU/g of Hb, P<0.001, respectively. There was a significant difference in the G6PD activity of RBC in patients' blastic and non-blastic phases, t (128) =-2.48, P=0.014. The G6PD activity of RBC is higher in childhood ALL than in the control group. Moreover, the G6PD activity of RBC in the blastic phase of leukemia was higher than that of patients in remission.

Recently, hematological parameters such as hemoglobin, white blood cell (WBC), mean platelet volume (MPV), and C-reactive protein (CRP) have received more attention as predictors of overweight/obesity among adolescents. We aimed to investigate the association between hemoglobin, WBC, MPV, and CRP and overweight/obesity among adolescents in two regions of Sudan: River Nile State in the north and Gadarif in the east. A multicenter community - based cross-sectional study was conducted from September 2022 to October 2023. A questionnaire was used to collect sociodemographic data. Weight, height, hematological parameters, and CRP were measured using standard procedures. Multivariate multinomial analysis was performed. A total of 738 adolescents (male: 325 [44.0%], female: 413 [56.0%]) were recruited. The median (interquartile, [IQR]) age was 14.8 (13.1-16.3) years. Of the total, 492 (66.7%), 151 (20.5%), and 95 (12.9%) were normal, underweight, and overweight/obese, respectively. In multivariate multinomial analysis, increasing WBC and increasing hemoglobin have shown a progressive increase in the overweight/obese group (adjusted odds ratio [AOR] = 1.08, 95% confidence interval [CI] 1.01-1.16) and (AOR = 1.27, 95% CI 1.06-1.53), respectively. Compared with females, males were at higher risk of being underweight (AOR = 2.77, 95% 1.86-4.12). This study indicates that the identified hematological predictors, specifically WBC and hemoglobin levels, can be helpful indicators for predicting overweight and obesity in adolescents in Sudan.

This study aims to investigate the etiologies of portal vein thrombosis (PVT) and Budd-Chiari syndrome (BCS), thereby enhancing improving early detection and management strategies for these conditions. A retrospective review was undertaken to identify the etiologies of PVT and BCS. A detailed clinical evaluation was performed and all underlying diseases, such as MPD, and related conditions (e.g. surgery) associated with thrombosis were recorded. The study comprised a total of 73 patients, with 58 diagnosed with PVT and 15 with BCS. Of these patients, 56 (76.7%) had at least one underlying disease. The most prevalent underlying diseases in patients with PVT were cirrhosis (32/58, 55.2%), myeloproliferative disease (3/58, 5.2%), malignancy (4/58, 6.9%), and rheumatological conditions (4/58, 6.9%). For BCS, 11/15 patients (73.3%) had at least one predisposing condition, including cirrhosis in six cases. Congenital causes were identified in 16/58 cases of PVT (27.6%), in 7/15 cases of BCS (46.7%). Thirty-two patients had previously undergone gastrointestinal surgery (PVT 24/58, BCS 8/15); surgery was the sole etiology in 15/73 patients (20.5%). Homocysteinemia was common (PVT 20/58, BCS 5/15). A multitude of rare etiologies were identified, including paroxysmal nocturnal haemoglobinuria, Crohn's disease, nephrotic syndrome, drug therapies, pregnancy, JAK2 mutation, and elevated factor VIII or fibrinogen. The presence of a wide range of diverse frequent-infrequent etiologies of congenital or acquired splanchnic vein thrombosis in this cohort underscores the necessity for the implementation of appropriate diagnostic strategies in a broad spectrum of at-risk patients.

Chronic lymphocytic leukemia (CLL) is a hematologic malignancy characterized by the excessive production of lymphocytes in the bone marrow. One of the emerging therapeutic strategies for CLL is chimeric antigen receptor (CAR) T-cell therapy, wherein T-cells are genetically modified to recognize and target cancer cells more effectively. The present study aims to systematically compare the therapeutic impact of high-dose versus low-dose status of CAR T-cell therapy targeting CD19 (CART-19) in patients with relapsed or refractory CLL. To identify relevant studies, a comprehensive literature search was conducted in PubMed, Scopus, and Web of Science databases up to April 2023. The primary outcome measures included treatment response rates, assessed as complete response (CR) and partial response (PR), and toxicity, as indicated by the incidence of cytokine release syndrome (CRS). Additionally, sensitivity and bias analyses were performed to evaluate the robustness of the findings. Four randomized controlled trials (RCTs) comprising 89 patients with relapsed or refractory CLL met the inclusion criteria. Comparison of treatment response rates between high-dose and low-dose CART-19 therapy demonstrated a significantly higher complete and partial response rate in the high-dose group (SMD [95% CI]: 1.02 [0.10, 1.94]; P<0.05). However, no significant association was observed between CTL019 dosage and the incidence of CRS (P>0.05). This meta-analysis suggests that high-dose CART-19 is associated with improved response rates and survival outcomes in patients with CLL compared to low-dose therapy. However, due to variability in study results, further large-scale, well-designed trials are required to establish the optimal therapeutic dosing strategy for CART-19 therapy in CLL.

Sitosterolemia is a rare inherited condition caused by elevated levels of plant sterols in the plasma, characterized by mutations in ABCG5 and ABCG8 genes. A scarce occurrence in this condition are hematological abnormalities such as hemolytic anemia, stomatocytosis, and macrothrombocytopenia. We conducted a meta-analysis and systematic review to answer these questions regarding patients who have hemolytic anemia and ABCG8 mutation. 13 reports were shortlisted for the final analysis (Observational studies-6, case series-4, case reports-3). Descriptive statistics were utilized to study the patient characteristics. From the 13 reports that we found in available literature, we identified 19 cases of ABCG8 mutation and anemia. From the random-effects proportions model, the chance of this event occurring among patients with sitosterolemia was 6.8% [0.068, 95% Confidence Interval (CI) 0.016-0.120, P=0.010] (I2 24.68%) (14/145). Thrombocytopenia and stomatocytosis were frequently reported. Splenomegaly and xanthomas were other common associations. To the best of our knowledge, we provide the first report of the prevalence of anemia, specifically in patients with sitosterolemia caused by a mutation in the ABCG8 gene. At 6.8%, this is an extremely rare occurrence in an already infrequent disease.

We report a novel α-globin gene variant, hemoglobin (Hb) Móstoles, characterized by a single nucleotide substitution in the HBA2 gene [α2 58(E7) His > Arg; HBA2:c.176A>G], associated with a 3.7-kb deletion in the homologous chromosome. This variant was identified in a Moroccan family living in Spain. The proband, a four-year-old girl, presented with microcytosis and hypochromia. The abnormal Hb was maternally inherited and detected in two of the proband's four siblings, while the 3.7-kb deletion was paternally inherited. Hb analysis using high-performance liquid chromatography and capillary electrophoresis revealed an abnormal peak in the Hb S region, with a concentration of approximately 3%. Hematological parameter assessment of the four carriers demonstrated that, despite being a structural hemoglobinopathy, Hb Móstoles is associated with an alpha-thalassemia phenotype and may exacerbate clinical manifestations when coexisting with other HBA1 and HBA2 mutations.